ポイツ・ジェガース症候群
WordNet
- a small vascular growth on the surface of a mucous membrane (同)polypus
- one of two forms that coelenterates take (e.g. a hydra or coral): usually sedentary with a hollow cylindrical body usually with a ring of tentacles around the mouth; "in some species of coelenterate, polyps are a phase in the life cycle that alternates with a medusoid phase"
PrepTutorEJDIC
- ポリプ(ヒドラ,イソギンチャクなどの生物)・ポリープ(鼻などの粘膜にできるおでき)
- =polytechnic
UpToDate Contents
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English Journal
- Colorectal polyps in childhood.
- Thakkar K, Fishman DS, Gilger MA.SourceThe Section of Pediatric Gastroenterology, Hepatology and Nutrition at Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA.
- Current opinion in pediatrics.Curr Opin Pediatr.2012 Aug 9. [Epub ahead of print]
- PURPOSE OF REVIEW: Colorectal polyps are a common cause of gastrointestinal bleeding in children. This review updates the information on colorectal polyps and summarizes the recent advances in genetics, diagnosis, and treatment of polyps in the large intestine.RECENT FINDINGS: A review of recent lit
- PMID 22890064
- Contribution of capsule endoscopy to Peutz-Jeghers syndrome management in children.
- Gastineau S, Viala J, Caldari D, Mas E, Darviot E, Le Gall C, Maurage C, Michaud L, Dabadie A.SourceCHU Rennes, Department of Pediatrics, South Hospital, Rennes cedex, France.
- Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver.Dig Liver Dis.2012 Jul 12. [Epub ahead of print]
- BACKGROUND: Capsule endoscopy is recommended for children with Peutz-Jeghers syndrome as young as 8 years of age. Aim of our study was to evaluate the contribution of capsule endoscopy in managing risk of further obstructive complications.METHODS: A retrospective analysis of 27 children who received
- PMID 22795616
Japanese Journal
- Resection of Over 290 Polyps During Emergency Surgery for Four Intussusceptions with Peutz-Jeghers Syndrome : Report of a Case
- AKIMARU KOHO,KATOH SHUJI,ISHIGURO SHINGO,MIYAKE KENJI,SHIMANUKI KIMIYOSHI,TAJIRI TAKASHI
- Surgery today : the Japanese journal of surgery 36(11), 997-1002, 2006-11-01
- NAID 10018814364
- 筋上皮性過誤腫を先進部とした回腸・回腸型腸重積症例の1例
- 岩崎 稔,山本 栄司,田中 紘一
- 日本小児外科学会雑誌 40(6), 806-811, 2004-10-20
- 症例は生後4ヵ月の女児である.嘔吐を主訴に来院し,来院時には中等度以上の脱水と活力の低下,腹部膨満を認めた.浣腸を施行したところ,多量の粘血便を認め,腹部超音波検査,腹部・骨盤部CT検査の結果,回腸・回腸型腸重積症が示唆され,緊急開腹手術を施行した.摘出標本による病理組織学的診断結果によると,筋上皮性過誤腫に誘発された回腸・回腸型腸重積症と判明した.筋上皮性過誤腫は,症例報告も僅かに散見される程度 …
- NAID 110002129842
Related Links
- Offers information on Peutz Jeghers Syndrome, this rare disorder characterized by hamartomas and high risk for cancer. For patients, family, and friends. Peutz-Jeghers Syndrome Peutz-Jeghers Syndrome (PJS), a rare disorder that ...
- Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with a distinct pattern of skin and mucosal macular melanin deposition. Patients with Peutz-Jeghers syndrome have a 15-fold increased risk of developing intestinal ...
- Nasal Polyps / no comments I’ve had plenty of bloody noses throughout my life, but think they are more to do with my inability to hold onto my life force. In my mind they are connected with anemia from polyps and periods, diarreah ...
Related Pictures
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- 英
- Peutz-Jeghers syndrome, PJS
- 同
- ポイツ・イェガース症候群 ポイツ・イェガー症候群 Peutz-Jeghers症候群、過誤腫性大腸ポリポーシス、口唇掌蹠母斑腸管ポリポーシス
- 関
- Peutz-Jeghers polyp Peutz-Jeghers polyps
- 大腸ポリポーシス、消化管ポリポーシス、神経皮膚症候群、母斑症
- familial polyposis syndrome
概念
- 口唇の黒褐色色素沈着と消化管ポリープを主徴とする症候群。
病型
病因
- germ-line mutations in the LKB1 gene
- 遺伝子変異:19p13.3 STK11, LKB1
疫学
遺伝形式
病理
- 過誤腫性ポリープ:粘膜筋板の樹枝状増生、腺管の延長と増生など。樹枝状に分枝してており、ポリープの茎が太いため腸重積をきたしやすい。
症状
- 初発症状:口唇色素沈着、腹痛、消化管出血、腸重積
- 皮膚粘膜症状
-
- 色素沈着は表皮基底層におけるメラニン細胞の増殖とメラニン色素沈着による。思春期以降は退色。
合併症
- 消化管、皮膚、卵巣、子宮。ポリープ自体の癌化率は低いが、消化器、性腺など他の部位の癌化率は高い(QB.A-398)。
- 一生涯の腫瘍発生率は37-93%、相対危険度は10-18。悪性腫瘍が派生する平均年齢は42歳。好発部位は結直腸癌、乳癌、胃癌、小腸癌、膵癌の順に多い。(1644人の患者を対象とした20のコホート研究のシステミックレビューによる)(参考1)
検査
診断
治療
手術療法
- 適応:ポリープが原因により出血、イレウスをきたした場合。腺腫合併例や癌化例。
予後
- 大腸に生じたものは癌化しやすい
- a benign polyposis syndrome is not a risk factor for colorectal cancer (first aid step1 2006 p.277)
- 消化管外の悪性腫瘍の発生リスクが上昇する
参考
- 1. PEUTZ-JEGHERS SYNDROME; PJS - OMIM
- http://omim.org/entry/175200
- 2. [charged] Overview of Peutz-Jeghers syndrome - uptodate [1]
- 3. [charged] Peutz-Jeghers syndrome and juvenile polyposis: Screening and management of patients and families - uptodate [2]
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- 関
- multi